Aplasia cutis congenita

Moises Salama, Latanya T. Benjamin, Seth R. Thaller, Lawrence A. Schachner

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Aplasia cutis congenita (ACC) denotes congenitally localized or widespread absence of skin (1,2). Multiple classification systems have been proposed (3) for this heterogeneous group of disorders, which is associated with various other anomalies. Frieden (4) developed a nine-category classification system to group distinct clinical subtypes of ACC. The criteria for distinction between the groups takes into account the location and pattern of skin absence, associated malformations, and the mode of inheritance (4). Characteristically, the defect is a midline solitary lesion that most commonly affects the scalp (86%) without associated anomalies. Sometimes, this defect of the integumentary system extends beyond the dermis to involve the underlying bony calvarium and dura, occurring in approximately 35% of the scalp defect cases (5). Although the true incidence is unknown, ACC is a rare condition believed to occur in about one in 3000 live births (6). Since Cordon’s original article in 1767 (2), greater than 500 cases, have been reported. Cordon’s article was the first to describe ACC of the extremities, followed in 1826 by Campbell who described defects involving the scalp (7).

Original languageEnglish (US)
Title of host publicationSoft-Tissue Surgery of the Craniofacial Region
PublisherCRC Press
Pages345-349
Number of pages5
ISBN (Electronic)9781420016789
ISBN (Print)9780824728939
StatePublished - Jan 1 2007

Fingerprint

Ectodermal Dysplasia
Scalp
Integumentary System
Skin
Live Birth
Dermis
Skull
Extremities
Incidence

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Salama, M., Benjamin, L. T., Thaller, S. R., & Schachner, L. A. (2007). Aplasia cutis congenita. In Soft-Tissue Surgery of the Craniofacial Region (pp. 345-349). CRC Press.

Aplasia cutis congenita. / Salama, Moises; Benjamin, Latanya T.; Thaller, Seth R.; Schachner, Lawrence A.

Soft-Tissue Surgery of the Craniofacial Region. CRC Press, 2007. p. 345-349.

Research output: Chapter in Book/Report/Conference proceedingChapter

Salama, M, Benjamin, LT, Thaller, SR & Schachner, LA 2007, Aplasia cutis congenita. in Soft-Tissue Surgery of the Craniofacial Region. CRC Press, pp. 345-349.
Salama M, Benjamin LT, Thaller SR, Schachner LA. Aplasia cutis congenita. In Soft-Tissue Surgery of the Craniofacial Region. CRC Press. 2007. p. 345-349
Salama, Moises ; Benjamin, Latanya T. ; Thaller, Seth R. ; Schachner, Lawrence A. / Aplasia cutis congenita. Soft-Tissue Surgery of the Craniofacial Region. CRC Press, 2007. pp. 345-349
@inbook{f818d0c77c2a4b6f90e39908c86ca6fd,
title = "Aplasia cutis congenita",
abstract = "Aplasia cutis congenita (ACC) denotes congenitally localized or widespread absence of skin (1,2). Multiple classification systems have been proposed (3) for this heterogeneous group of disorders, which is associated with various other anomalies. Frieden (4) developed a nine-category classification system to group distinct clinical subtypes of ACC. The criteria for distinction between the groups takes into account the location and pattern of skin absence, associated malformations, and the mode of inheritance (4). Characteristically, the defect is a midline solitary lesion that most commonly affects the scalp (86{\%}) without associated anomalies. Sometimes, this defect of the integumentary system extends beyond the dermis to involve the underlying bony calvarium and dura, occurring in approximately 35{\%} of the scalp defect cases (5). Although the true incidence is unknown, ACC is a rare condition believed to occur in about one in 3000 live births (6). Since Cordon’s original article in 1767 (2), greater than 500 cases, have been reported. Cordon’s article was the first to describe ACC of the extremities, followed in 1826 by Campbell who described defects involving the scalp (7).",
author = "Moises Salama and Benjamin, {Latanya T.} and Thaller, {Seth R.} and Schachner, {Lawrence A.}",
year = "2007",
month = "1",
day = "1",
language = "English (US)",
isbn = "9780824728939",
pages = "345--349",
booktitle = "Soft-Tissue Surgery of the Craniofacial Region",
publisher = "CRC Press",

}

TY - CHAP

T1 - Aplasia cutis congenita

AU - Salama, Moises

AU - Benjamin, Latanya T.

AU - Thaller, Seth R.

AU - Schachner, Lawrence A.

PY - 2007/1/1

Y1 - 2007/1/1

N2 - Aplasia cutis congenita (ACC) denotes congenitally localized or widespread absence of skin (1,2). Multiple classification systems have been proposed (3) for this heterogeneous group of disorders, which is associated with various other anomalies. Frieden (4) developed a nine-category classification system to group distinct clinical subtypes of ACC. The criteria for distinction between the groups takes into account the location and pattern of skin absence, associated malformations, and the mode of inheritance (4). Characteristically, the defect is a midline solitary lesion that most commonly affects the scalp (86%) without associated anomalies. Sometimes, this defect of the integumentary system extends beyond the dermis to involve the underlying bony calvarium and dura, occurring in approximately 35% of the scalp defect cases (5). Although the true incidence is unknown, ACC is a rare condition believed to occur in about one in 3000 live births (6). Since Cordon’s original article in 1767 (2), greater than 500 cases, have been reported. Cordon’s article was the first to describe ACC of the extremities, followed in 1826 by Campbell who described defects involving the scalp (7).

AB - Aplasia cutis congenita (ACC) denotes congenitally localized or widespread absence of skin (1,2). Multiple classification systems have been proposed (3) for this heterogeneous group of disorders, which is associated with various other anomalies. Frieden (4) developed a nine-category classification system to group distinct clinical subtypes of ACC. The criteria for distinction between the groups takes into account the location and pattern of skin absence, associated malformations, and the mode of inheritance (4). Characteristically, the defect is a midline solitary lesion that most commonly affects the scalp (86%) without associated anomalies. Sometimes, this defect of the integumentary system extends beyond the dermis to involve the underlying bony calvarium and dura, occurring in approximately 35% of the scalp defect cases (5). Although the true incidence is unknown, ACC is a rare condition believed to occur in about one in 3000 live births (6). Since Cordon’s original article in 1767 (2), greater than 500 cases, have been reported. Cordon’s article was the first to describe ACC of the extremities, followed in 1826 by Campbell who described defects involving the scalp (7).

UR - http://www.scopus.com/inward/record.url?scp=85057226877&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85057226877&partnerID=8YFLogxK

M3 - Chapter

AN - SCOPUS:85057226877

SN - 9780824728939

SP - 345

EP - 349

BT - Soft-Tissue Surgery of the Craniofacial Region

PB - CRC Press

ER -